WEEK14 Flashcards
(49 cards)
What are the two main categories of platelet disorders?
Quantitative and Qualitative
Quantitative disorders involve the number of platelets, while qualitative disorders involve the function of platelets.
What causes decreased platelet production?
Decreased platelet production can be due to:
* Abnormal platelet distribution
* Increased platelet destruction
* Pathophysiologic processes resulting in thrombocytopenia
What are common clinical manifestations of platelet and vascular disorders?
Common manifestations include:
* Ecchymoses
* Petechiae
* Purpura
* Mucosal bleeding
* Epistaxis
* Gingival bleeding
* Menorrhagia
* Hematuria
What is Congenital Amegakaryocytic Thrombocytopenia (CAMT)?
CAMT is characterized by:
* Mutation in the MPL gene
* Severe thrombocytopenia
* Absence of megakaryocytes in the bone marrow
* Markedly elevated serum thrombopoietin
What distinguishes CAMT 1 from CAMT 2?
CAMT 1 is associated with a more severe type of thrombocytopenia and early onset of pancytopenia, while CAMT 2 shows transient increases in platelet counts during the first year of life.
What mutation causes Autosomal Dominant Thrombocytopenia?
Mutation on ANKRD26 on chromosome 10, leading to incomplete megakaryocyte differentiation.
What is Wiskott-Aldrich Syndrome?
An X-linked hereditary disorder associated with:
* Combined immunodeficiency
* Thrombocytopenia
* Small platelets
* Eczema
* Increased risk of autoimmune disorders and cancer
What is the most common infectious agent causing Neonatal Thrombocytopenia?
Cytomegalovirus (CMV)
What are the two types of Immune Thrombocytopenic Purpura (ITP)?
Acute ITP and Chronic ITP
What is a key characteristic of Acute ITP?
Typically presents in young children with immune thrombocytopenia developing suddenly, with a platelet count of less than 20 X 10^9/L.
What is a characteristic finding in Chronic ITP?
Commonly presents in adults aged 20-50 years with platelet counts typically less than 30 X 10^9/L.
What is the treatment of choice for Chronic ITP?
IVIG (Intravenous Immunoglobulin)
What is the role of splenectomy in ITP treatment?
Splenectomy is a conventional therapy for ITP, especially in patients not responding to other treatments.
What is the most common drug associated with drug-induced thrombocytopenia?
Quinine
What is Neonatal Alloimmune Thrombocytopenia?
A condition resulting from immunization of the mother by fetal platelet antigen, leading to maternal antibody transfer.
What is HELLP Syndrome?
A disorder related to preeclampsia/eclampsia characterized by hemolysis, elevated liver enzymes, and low platelet count.
What are the typical findings in Thrombotic Thrombocytopenic Purpura (TTP)?
Includes:
* Thrombocytopenia
* Microangiopathic hemolytic anemia
* Fever
* Neurologic abnormalities
* Renal dysfunction
What distinguishes DIC from TTP in laboratory findings?
Coagulation screening tests and D-Dimer assay are normal in TTP but abnormal in DIC.
What are the two forms of Disseminated Intravascular Coagulation (DIC)?
Acute DIC and Chronic DIC
What is Kasabach-Merritt Syndrome?
A condition causing profound thrombocytopenia related to platelet trapping within a vascular tumor.
What characterizes Primary/Essential Thrombocytosis?
An increase in platelets due to clonal proliferation affecting all hematopoietic cells, often leading to bleeding tendencies.
What is Reactive/Secondary Thrombocytosis associated with?
Chronic inflammatory disease and iron deficiency anemia (IDA) associated with chronic blood loss.
What is the clonal proliferation that affects all hematopoietic cells?
Affects all hematopoietic cells
This condition can lead to bleeding tendencies due to platelet function abnormalities.
Which disorders are most commonly associated with bleeding tendencies?
- Hodgkin’s disease
- Polycythemia vera
- Myelofibrosis
- CML
- Thrombocythemia
